Predictors of Long-Term Outcomes in Hypertrophic Cardiomyopathy: The NHLBI HCM Registry.
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HCMR Investigators, Kramer Christopher M, Kolm Paul, DiMarco John P, Desai Milind Y, Ho Carolyn Y, Kwong Raymond Y, Dolman Sarahfaye F, Desvigne-Nickens Patrice, Geller Nancy, Kim Dong-Yun, Schulz-Menger Jeanette, Friedrich Matthias G, Maron Martin S, Appelbaum Evan, Link Mark S, Francis Gary S, Greenberg Barry, Jerosch-Herold Michael, Piechnik Stefan, Mahmod Masliza, Raman Betty, Jacoby Daniel L, Baldassare Lauren A, White James A, Chiribiri Amedeo, Helms Adam S, Choudhury Lubna, Michels Michelle, Bradlow William M, Salerno Michael, Heitner Steven B, Masri Ahmad, Prasad Sanjay K, Mohiddin Saidi A, Plein Sven, Madias Christopher, Mahrholdt Heiko, Bucciarelli-Ducci Chiara, Nightingale Angus K, Weinsaft Jonathan W, Kim Han W, McCann Gerry P, van Rossum Albert, Germans Tjeerd, Williamson Eric E, Geske Jeffrey B, Flett Andrew S, Dawson Dana, Mongeon Francois-Pierre, Olivotto Iacopo, Crean Andrew M, Woo Anna, Owens Anjali T, Anderson Lisa, Sharma Sanjay, Biagini Elena, Newby David E, Andre Florian, Berry Colin, Kim Bette, Larose Eric, Abraham Theodore P, Hays Allison G, Sherrid Mark V, Gelfand Eli V, Nagueh Sherif F, Rimoldi Ornella, Camici Paolo, Elstein Eleanor, Autore Camillo, Watkins Hugh, Weintraub William S, Neubauer Stefan
Аннотация
OBJECTIVE: To combine prospectively collected clinical history, imaging, genetic, and biomarker data to improve risk prediction of adverse events in hypertrophic cardiomyopathy.
DESIGN, SETTING, AND PARTICIPANTS: A total of 2750 patients with hypertrophic cardiomyopathy were prospectively enrolled in the registry-based study from 44 sites in North America and Europe with expertise in hypertrophic cardiomyopathy and cardiac magnetic resonance (CMR) imaging. Participants were enrolled from April 1, 2014, to April 7, 2017.
EXPOSURES: Patients underwent a health history questionnaire, blood sampling for biomarkers and genotyping, and contrast-enhanced CMR. Patients were followed up yearly by telephone and through records review regarding event documentation.
MAIN OUTCOMES AND MEASURES: The predefined composite adjudicated primary end point was time to first event for hypertrophic cardiomyopathy-related deaths; nonfatal sustained ventricular arrhythmias (VAs) requiring cardioversion or defibrillation; and left ventricular (LV) assist device implant or heart transplant. A secondary end point was a composite of sudden cardiac death and nonfatal VA events. The elastic-net method identified the most important predictors. Cox proportional hazards regression assessed associations with time to the first end point.
RESULTS: Of the 2750 prospectively enrolled patients, 2698 (98%) had analyzable data after 9 were excluded because they had hypertrophic cardiomyopathy phenocopies and 43 withdrew. Of these remaining patients, 1919 (71%) were male, mean age was 50 years (SD, 11 years), and 423 (16%) were from underrepresented racial and minority groups. The mean follow-up was 6.9 years (SD, 2.1 years). The primary event model in 104 patients included LV scar as a percentage of LV mass by late gadolinium enhancement (LGE%; hazard ratio [HR], 1.86; 95% CI, 1.58-2.20; P < .001), LV mass index (HR, 1.09; 95% CI, 1.01-1.17; P = .03), LV end-systolic volume index (HR, 1.28; 95% CI, 1.12-1.46; P < .001 ), all per 10-unit increase, history of heart failure at study entry (HR, 2.89; 95% CI, 1.75-4.77; P < .001), and log N-terminal pro-B-type natriuretic peptide (NT-proBNP; HR, 1.41; 95% CI, 1.17-1.70; P < .001) level per log unit, (C index for all, 0.77). An LGE percentage of the LV mass of 9% or higher substantially increased the primary composite event rate (P = .001). The secondary sudden cardiac death and VA risk factor model (in 69 patients) included LGE%, LV mass index, LV ejection fraction, and log(NT-proBNP) (C index, 0.76).
CONCLUSIONS AND RELEVANCE: These results provide prospective evidence for incorporating cardiac magnetic resonance and NT-proBNP in the evaluation of patients with hypertrophic cardiomyopathy.
TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT01915615.